Advances in pathogenic concepts and therapeutic agents in Rasmussen's encephalitis

Rasmussen's encephalitis is a rare inflammatory brain disease which occurs mainly in children and is characterised by affection of only one hemisphere. Pathogenetic concepts have considered three different, not mutually exclusive, key factors contributing to the initiating or perpetuating events in the central nervous system. These include viruses, autoimmune antibodies and autoimmune cytotoxic T lymphocytes. Based on these concepts, different therapeutic strategies have been pursued, such as antiviral agents, plasmapheresis, immuno-adsorption, immunosuppression or immunomodulation with intravenous immunoglobulins. However, due to the lack of larger studies, to date there is no established therapeutic strategy of this devastating disease. An overview of the current state of immunepathogenic concepts for Rasmussen's encephalitis is given and past and present treatment attempts are discussed, including an outline of future perspectives. An opinion on symptomatic treatment with anticonvulsive drugs is included.